A comparison between childhood and adult onset systemic lupus erythematosus adjusted for ethnicity from the 1000 Canadian Faces of Lupus Cohort

Rheumatology ◽  
2019 ◽  
Vol 58 (8) ◽  
pp. 1393-1399 ◽  
Author(s):  
Hyein Kim ◽  
Deborah M Levy ◽  
Earl D Silverman ◽  
Carol Hitchon ◽  
Sasha Bernatsky ◽  
...  

Abstract Objective Childhood-onset SLE (cSLE) manifests differently than adult-onset SLE (aSLE). This study determined whether ethnic differences contribute to the differences in clinical presentation between the two groups. Methods This cross-sectional study used data from a multi-centred registry from eight adult and four paediatric Canadian centres gathered at study entry. We compared the frequency of clinical manifestations and autoantibodies between aSLE and cSLE. For those with a significant difference, a multivariable logistic regression was performed, adjusting for ethnicity, SLE onset (cSLE vs aSLE), disease duration and centre. Disease activity and damage between aSLE and cSLE were compared after stratifying by disease duration. Results Of 552 aSLE subjects, 502 (90.9%) were female and 381 (69.0%) were Caucasian. Mean age at diagnosis was 37.0 ± 13.6 years and disease duration 10.9 ± 9.6 years. Of 276 cSLE subjects, 231 (83.7%) were female and 101 (36.6%) were Caucasian. Mean age at diagnosis was 12.7 ± 3.3 years and disease duration 5.6 ± 8.2 years. In multivariable regression analysis, aSLE was associated with decreased odds of having a neurologic disorder (odds ratio = 0.49) and increased odds of having aCL antibodies (odds ratio = 1.85). Disease activity and damage accrual scores were higher in aSLE than cSLE within the same disease duration strata, although the differences were not clinically significant. Ethnicity was not associated with any differences in clinical manifestations or autoantibody frequency between aSLE and cSLE. Conclusion Although a crude comparison of aSLE and cSLE yielded several differences in clinical symptoms and autoantibodies, this difference was not attributable to ethnic differences between aSLE and cSLE.

2021 ◽  
Vol 10 (2) ◽  
pp. 243
Author(s):  
Matteo Piga ◽  
Laurent Arnaud

Systemic lupus erythematosus (SLE) is an immune-mediated multi-systemic disease characterized by a wide variability of clinical manifestations and a course frequently subject to unpredictable flares. Despite significant advances in the understanding of the pathophysiology and optimization of medical care, patients with SLE still have significant mortality and carry a risk of progressive organ damage accrual and reduced health-related quality of life. New tools allow earlier classification of SLE, whereas tailored early intervention and treatment strategies targeted to clinical remission or low disease activity could offer the opportunity to reduce damage, thus improving long-term outcomes. Nevertheless, the early diagnosis of SLE is still an unmet need for many patients. Further disentangling the SLE susceptibility and complex pathogenesis will allow to identify more accurate biomarkers and implement new ways to measure disease activity. This could represent a major step forward to find new trials modalities for developing new drugs, optimizing the use of currently available therapeutics and minimizing glucocorticoids. Preventing and treating comorbidities in SLE, improving the management of hard-to-treat manifestations including management of SLE during pregnancy are among the remaining major unmet needs. This review provides insights and a research agenda for the main challenges in SLE.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
O Gacem ◽  
L Labboun ◽  
N Mansouri ◽  
M Gherbi ◽  
Z Zeroual ◽  
...  

Abstract Background Pediatric Systemic Lupus Erythematosus (pSLE) is a chronic mutisystemic autoimmune disease with complex clinical manifestations whose diagnosis is not always easy and the course is generally severe and the treatment is not very well codified and often extrapolated from that of adults. This study aims to describe the clinical, immunological, therapeutic characteristics and short outcome of systemic lupus erythematosus in Algerian children. Methods This was a prospective, multicentre and descriptive study 36 months (January 2015 - December 2018) at the department of Pediatrics of University Hospital Nefissa Hamoud ex Parnet Algiers. Children less than16 years of age fulfilling the American College of Rheumatology SLE criteria were included. Disease activity estimated by Systemic Lupus Erythematosus Disease Activity index (SLEDAI) whose use has been validated in children and damage index based on Systemic Lupus International Collaborating Clinics (SLICC) score were determined. Results Eighty-three (83) patients were studied. Female: male ratio was1:49. Mean ages at lupus onset and diagnosis were respectively: 10, 12 ± 3, 88 and 11, 3 ± 3, 62 years. All patients had skin involvement while constitutional signs including fever and asthenia were observed in (98.8%). Rheumatological, renal, neuropsychiatric, cardiac, hepato-digestive, pleuropulmonary and ocular disorders were observed respectively: 65, 1%, 44, 6%, 41%, 27, 7%, 41%, 19, 3% and 7, 2%. All patients were positive for antinuclear antibodies. Anti-double-stranded DNA (75%) was the most frequently observed autoantibody profile. Antiphospholipid antibody positivity was noted in 52% whereas hypocomplementemia in fractions C3, C4 was observed in 55% and 56% respectively. In our study, the severe forms were more frequent (83%) than the mild ones (17%) with a significant difference (P = < 10–6). Overall, the mean SLEDAI at disease onset was 22.11 ± 11.87 with high activity ≥ 20 in 59% of cases. The mean damage score was 1.8 ± 2.045 (interquartile range 0–8). Among induction drugs, oral corticosteroids were the most frequently used (92%), and in a third of cases intravenously at high doses in combination with immunosuppressive therapy. In induction therapy, cyclophosphamide (CYC) was the most used drug (23%) compared with mycophenolate mofetil (MMF) (14%). Unlike the maintenance phase where MMF observed an increase (28%) vs (8%) CYC. The use of MMF was correlated with severe lupus nephritis with a significantly effective difference in the decrease in SLEDAI (P = 0.0001). The use of hydroxychloroquine (HCQ) was observed in 81% in induction and 89% in maintenance treatment. The correlation of HCQ use with survival was significantly positive (P = 0.04). Indeed, adherence to treatments and essentially HCQ was a protective factor, its odds ratio is < 1 with a significant p-value, [OR 0.016 95% CI (0.001–0.353)]. Mortality was estimated at 11%. Multivariable regression analysis showed that the neurological involvement (odds ratio = 6,093 95% confidence interval ((1,1 8 0 ∼ 31 446)) and macrophage activation syndrome were associated with a high risk of mortality. Conclusion we report a series of pSLE characterized by great clinical and biological heterogeneity. It follows a severe course of the disease with high disease activity at the diagnosis and therefore leads to high morbidity and mortality. However, these results must be confirmed by other pediatric studies which could form the basis of a diagnostic and therapeutic approach more adapted for children. Keywords Algeria, Child, Clinical features, Disease activity, lupus


2020 ◽  
Author(s):  
Worawit Louthrenoo ◽  
Thananant Trongkamolthum ◽  
Nuntana Kasitatnon ◽  
Antika Wongthanee

Abstract Background: Flares in pregnant patients with systemic lupus erythematosus (SLE) have shown conflicting results. This study aimed to determine the disease activity, and rate and severity of flares in pregnant SLE patients compared with the non-pregnant SLE controls. Methods: The medical records of pregnant patients in the SLE cohort were identified. Controls were non-pregnant female SLE patients who were matched for age at diagnosis and disease duration prior to conception. Disease activity was determined by mSLEDAI-2K. The definition and severity of flares followed the SELANA-SLEDAI Flare Index. The disease activity was measured from 6 months prior to conception (-6M) until termination of pregnancy or delivery.Results: Ninety pregnancies occurred from 77 patients, of whom 36.67% had active disease at the time of conception. The pregnancy group was slightly, but significantly, younger than the control group at diagnosis (21.63 ± 5.89 years vs. 24.05 ± 7.27 years, p = 0.015). The SLE disease activity (mSLEDAI-2K) score was comparable in both groups from -6M to the post-partum period, with that in the control group being slightly but significantly higher than that in the pregnancy group at conception (3.57 ± 4.28 vs. 1.91 ± 3.44, p = 0.003). Of the 90 pregnancies, the overall incidence of flares in both groups was similar during pregnancy (39 vs. 26, p = 0.070). There also was no difference in the incidence of flare during each trimester, with 19 vs.7 flares among 90 pregnancies (p = 0.588) in the 1st, 12 vs. 12 among 82 (p = 0.682) in the 2nd, and 6 vs. 7 among 71 (p = 0.266) in the 3rd trimester. The incidence of flare during the post-partum period also was similar (42 vs. 30 flares among 90 pregnancies, p = 0.091). There was no difference in the severity of flare in each trimester, overall flare during pregnancy or post-partum flare. Conclusions: This study found no difference in flare rate or severity of flares between pregnant SLE patients and non-pregnant SLE controls, who were matched by sex, age at diagnosis and disease duration prior to pregnancy.


Lupus ◽  
2019 ◽  
Vol 28 (9) ◽  
pp. 1101-1110 ◽  
Author(s):  
V R Pimentel-Quiroz ◽  
M F Ugarte-Gil ◽  
GB Harvey ◽  
D Wojdyla ◽  
G J Pons-Estel ◽  
...  

Aim The aim of this study was to identify factors predictive of serious infections over time in patients with systemic lupus erythematosus (SLE). Methods A multi-ethnic, multi-national Latin American SLE cohort was studied. Serious infection was defined as one that required hospitalization, occurred during a hospitalization or led to death. Potential predictors included were sociodemographic factors, clinical manifestations (per organ involved, lymphopenia and leukopenia, independently) and previous infections at baseline. Disease activity (SLEDAI), damage (SLICC/ACR Damage Index), non-serious infections, glucocorticoids, antimalarials (users and non-users), and immunosuppressive drugs use; the last six variables were examined as time-dependent covariates. Cox regression models were used to evaluate the predictors of serious infections using a backward elimination procedure. Univariable and multivariable analyses were performed. Results Of the 1243 patients included, 1116 (89.8%) were female. The median (interquartile range) age at diagnosis and follow-up time were 27 (20–37) years and 47.8 (17.9–68.6) months, respectively. The incidence rate of serious infections was 3.8 cases per 100 person-years. Antimalarial use (hazard ratio: 0.69; 95% confidence interval (CI): 0.48–0.99; p = 0.0440) was protective, while doses of prednisone >15 and ≤60 mg/day (hazard ratio: 4.18; 95 %CI: 1.69–10.31; p = 0.0019) and >60 mg/day (hazard ratio: 4.71; 95% CI: 1.35–16.49; p = 0.0153), use of methylprednisolone pulses (hazard ratio: 1.53; 95% CI: 1.10–2.13; p = 0.0124), increase in disease activity (hazard ratio: 1.03; 95% CI: 1.01–1.04; p = 0.0016) and damage accrual (hazard ratio: 1.22; 95% CI: 1.11–1.34; p < 0.0001) were predictive factors of serious infections. Conclusions Over time, prednisone doses higher than 15 mg/day, use of methylprednisolone pulses, increase in disease activity and damage accrual were predictive of infections, whereas antimalarial use was protective against them in SLE patients.


Reumatismo ◽  
2018 ◽  
Vol 70 (2) ◽  
pp. 85 ◽  
Author(s):  
Y. Emad ◽  
T. Gheita ◽  
H. Darweesh ◽  
P. Klooster ◽  
R. Gamal ◽  
...  

The aim was to explore possible correlations of antibodies to extractable nuclear antigens (ENA) with clinical manifestations and disease activity indices in systemic lupus erythematosus (SLE) patients. A total of 70 consecutive SLE patients (64 females) were included. Disease activity was assessed by SLE activity index (SLEDAI), and British Isles Lupus Assessment Group (BILAG). Anti-Ro/SSA correlated positively with, headache (r=0.24, p=0.04), blurring of vision (r=0.25, p=0.03) and SLEDAI (r=0.25, p=0.04) and negatively with C3 (r=–0.35, p=0.003). Anti-Ro/SSA correlated with anti La/SSB antibodies (r=0.69, p<0.001), but not with anti-DNA, anti-RNP and anti-Sm antibodies. Anti-La/SSB antibodies correlated with headache (r=0.26, p=0.03), SLEDAI (r=0.25, p=0.03) and negatively with C3 (r=–0.34, p=0.004). Anti-La/SSB did not correlate with anti-RNP or anti-Sm antibodies. Anti-Sm antibodies correlated with disease duration (r=0.34, p=0.003), 24 hours urinary proteins (r=0.31, p=0.008), SLEDAI (r=0.31, p=0.009), BILAG renal score (r=0.29, p=0.02) and negatively with age at onset (r=–0.27, p=0.02), WBCs (r=–0.29, p=0.014) and C4 (r=–0.25, p=0.049). In multivariate analyses, anti-Ro/SSA antibodies remained associated with headache, blurring of vision and C3 and anti-La/SSB antibodies remained associated with C3 and with headache. Anti-Sm antibodies were independently associated with disease duration and total SLEDAI scores, while anti-RNP antibodies remained significantly associated with BILAG mucocutaneous scores only. Antibodies to ENAs are associated with clinical aspects of SLE and may play a role in the assessment of disease activity. Insight into these ENAs may lead to new approaches to diagnostic testing, accurate evaluation of disease activity and lead to target approach for SLE.


2006 ◽  
Vol 26 (5) ◽  
pp. 718-722 ◽  
Author(s):  
Luis M. Vilá ◽  
María J. Molina ◽  
Angel M. Mayor ◽  
José J. Cruz ◽  
Eddy Ríos-Olivares ◽  
...  

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 606.1-606
Author(s):  
H. Assunção ◽  
M. Rodrigues ◽  
A. R. Prata ◽  
J. A. P. Da Silva ◽  
L. Inês

Background:Patients with systemic lupus erythematosus (SLE) often require hospitalization. The cause of admission may vary, but active disease and infection are consistently reported as the main reasons for hospitalization and are associated with worse survival and damage accrual. Recent improvements in the standard of care, including minimization of glucocorticoid dose and more effective and safe immunosuppressive regimens, may have changed the incidence and risk factors for hospitalization due to these causes. Hence, it is useful to identify predictors of hospitalization to further reduce the risk of admission for disease activity and severe infection in patients with SLE.Objectives:To identify predictors of hospitalization in patients with SLE, according to the underlying cause.Methods:Patients with SLE fulfilling classification criteria (ACR’97 and/or SLICC), regularly followed at an academic lupus clinic from January 2009 to December 2020 and with at least two outpatient visits were included in this cohort study. Time to first hospitalization up to 120 months was identified separately for the following admission causes: (a) any cause; (b) active SLE; (c) infection. Predictors of hospitalization were sought through survival analysis, with distinct models for each of the major admission causes. Univariate analysis was performed using Kaplan-Meier curves and Log-Rank tests. Tested variables assessed at baseline included: gender; age at SLE onset; age; disease duration; SLE Disease Activity Index (SLEDAI-2K) score; ongoing antimalarial use; ongoing immunosuppressants; ongoing prednisolone daily dose; lupus nephritis up to baseline; SLICC Damage Index (SDI) score. Variables with p<0.1 were further tested in multivariate Cox regression models. Hazard ratios (HR) were determined with 95% confidence intervals (95%CI).Results:We included 398 patients (female: 86.2%, mean age: 41.2±15.1 years, mean disease duration: 10.1±9.2 years; previous lupus nephritis: 28.9%; mean SLEDAI-2K score: 3.4±2.7; ongoing antimalarials: 78.9%; ongoing immunosuppressant: 29.9%; ongoing prednisolone >7.5 mg/day: 17.1%; SDI score ≥1: 28.4%). During the follow-up period, 50.5%, 23.6% and 17.3% were hospitalized at least once for any cause, active SLE or infection, respectively.In the multivariate model, significant baseline predictors for hospitalization due to active disease were (table 1): SLEDAI-2K score >5; disease duration ≤2 years; ongoing immunosuppressants; SDI score ≥1. Baseline independent predictors of hospitalization for infection included (table 1): male gender; SDI score ≥1; ongoing antimalarials were protective.Table 1.Predictors of hospitalization in multivariate Cox regression according to the admission causePredictorsHospitalization for active SLEHospitalization for infectionSLEDAI-2K score >52.43 (1.53-3.88)n.s.SLE duration ≤2 years1.70 (1.04-2.77)n.s.Ongoing immunosuppressant1.91 (1.24-2.95)n.s.SDI score ≥11.82 (1.16-2.86)2.14 (1.33-3.45)Male gendern.s.2.19 (1.23-3.89)No antimalarial treatmentn.s.2.20 (1.34-3.60)Risk for each predictor reported as Hazard Ratio (95% Confidence Interval); n.s.: non-significantConclusion:Tight control of disease activity, prevention of damage accrual, and treatment with antimalarials may contribute to minimize the risk of hospitalization for these two major causes of admission in patients with SLE.Disclosure of Interests:None declared


Lupus ◽  
2022 ◽  
pp. 096120332110614
Author(s):  
Claudia Elera-Fitzcarrald ◽  
Cristina Reatégui-Sokolova ◽  
Rocío V Gamboa-Cárdenas ◽  
Mariela Medina ◽  
Francisco Zevallos ◽  
...  

Objectives This study aims to determine whether the MetS predicts damage accrual in SLE patients. Methods This longitudinal study was conducted in a cohort of consecutive SLE patients seen since 2012 at one single Peruvian institution. Patients had a baseline visit and then follow-up visits every 6 months. Patients with ≥ 2 visits were included. Evaluations included interview, medical records review, physical examination, and laboratory tests. Damage accrual was ascertained with the SLICC/ACR damage index (SDI) and disease activity with the SLEDAI-2K. Univariable and multivariable Cox-regression survival models were carried out to determine the risk of developing new damage. The multivariable model was adjusted for age at diagnosis; disease duration; socioeconomic status; SLEDAI; baseline SDI; the Charlson Comorbidity Index; daily dose; and time of exposure of prednisone (PDN), antimalarials, and immunosuppressive drugs. Results Two hundred and forty-nine patients were evaluated; 232 of them were women (93.2%). Their mean (SD) age at diagnosis was 35.8 (13.1) years; nearly all patients were Mestizo. Disease duration was 7.4 (6.6) years. The SLEDAI-2K was 5.2 (4.3) and the SDI, 0.9 (1.3). One hundred and eight patients (43.4%) had MetS at baseline. During follow-up, 116 (46.6%) patients accrued at least one new point in the SDI damage index. In multivariable analyses, the presence of MetS was a predictor of the development of new damage (HR: 1.54 (1.05–2.26); p < 0.029). Conclusions The presence of MetS predicts the development of new damage in SLE patients, despite other well-known risk factors for such occurrence.


Lupus ◽  
2019 ◽  
Vol 28 (11) ◽  
pp. 1344-1349
Author(s):  
C Reátegui-Sokolova ◽  
Z Rodríguez-Bellido ◽  
R V Gamboa-Cárdenas ◽  
M Medina ◽  
F Zevallos ◽  
...  

Objective The aim of this study was to determine whether remission and low disease activity state protect systemic lupus erythematosus patients from being hospitalized. Materials and methods Patients from the Almenara Lupus Cohort were included. Visits were performed every 6 months. Variables were measured at each visit. Hospitalizations were evaluated in the interval between two visits. Remission was defined as: a SLEDAI-2 K of 0, prednisone ≤5 mg/day and immunosuppressants on maintenance dose; low disease activity state as: a SLEDAI-2 K of ≤4, prednisone ≤7.5 mg/day and immunosuppressants on maintenance dose. Univariable and multivariable interval-censored survival regression models were used. In multivariable analysis, possible confounders were gender, age at diagnosis, socioeconomic status, educational level, disease duration, antimalarial use, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SDI) and Charlson comorbidity index. Confounders were determined in the same visit as disease activity state. Results Of the 308 patients, 92.5% of them ( n = 285) were women, had a mean age at diagnosis of 34.8 (13.4) years and a disease duration of 7.7 (6.5) years. At baseline the mean SDI was 1.13 (1.34). A total of 163 of the patients were hospitalized. In the multivariable analysis remission (hazard ratio 0.445 (0.274–0.725), P = 0.001) and low disease activity state (relative risk 0.504 (0.336–0.757), P = 0.001) at baseline were found to decrease the risk of hospitalization in systemic lupus erythematosus patients. A total of 158 hospitalizations presented a discernible cause. Disease activity was the most common cause of hospitalization, with 84 admissions (53.16%), the majority, 38, was due to active kidney disease (45.23%). Conclusion Remission and low disease activity state decreased the risk of hospitalizations in these systemic lupus erythematosus patients. Disease activity, particularly renal, was the most frequent cause of hospitalization.


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